Table 1.
Baseline characteristics of 94 LMNA genotype–positive patients, and comparison of patients with and without VA at time of censor
| All patients at time of inclusion (n = 94) | Patients with VA parameters at time of VA (n = 19) | Patients without VA at time of last follow up (n = 75) | P-value | |
|---|---|---|---|---|
| Age, years | 38 (27–51) | 47 (40–57) | 44 (28–54) | 0.04 |
| Proband, n (%) | 30 (32) | 10 (53) | 20 (27) | 0.03 |
| Female, n (%) | 50 (53) | 9 (47) | 41(55) | 0.57 |
| Non-missense mutation, n (%) | 81 (86) | 18 (95) | 63 (84) | 0.23 |
| Phenotype positive, n (%) | 77 (82%) | 19 (100) | 62 (83) | 0.06 |
| AV block,an (%) | 45 (52) | 17 (94) | 34 (50) | <0.001 |
| Atrial fibrillation, n (%) | 42 (45) | 16 (84) | 42 (56) | 0.03 |
| NSVT, n (%) | 30 (32) | 13 (68) | 37 (49) | 0.07 |
| Syncope, n (%) | 20 (22) | 7 (37) | 19 (26) | 0.27 |
| NYHA functional class ≥ II, n (%) | 26 (28) | 11 (61) | 23 (32) | 0.02 |
| Beta-blocker, n (%) | 18 (19) | 15 (79) | 47 (66) | 0.28 |
| ACE/AT2 inhibitor, n (%) | 18 (19) | 15 (79) | 27 (36) | 0.001 |
| MCRA, n (%) | 4 (4) | 4 (21) | 6 (8) | 0.21 |
| Cordarone/dronedarone, n (%) | 4 (4) | 5 (26) | 1 (1) | 0.001 |
| LVEF (%) | 51 ± 12 | 40 ± 12 | 51 ± 11 | <0.001 |
| GLS (%) | −16.1 ± 4.6 | −11.7 ± 4.3 | −15.6 ± 4.2 | 0.01 |
| LV EDVi (m) | 71 ± 19 | 83 ± 22 | 67 ± 16 | <0.001 |
| LV ESVi (mL) | 36 ± 17 | 52 ± 20 | 34 ± 16 | <0.001 |
| LAVi (mL) | 41 ± 19 | 70 ± 24 | 41 ± 15 | <0.001 |
| RVD (mm) | 41 ± 8 | 47 ± 10 | 42 ± 7 | 0.03 |
| TAPSE (mm) | 23 ± 5 | 20 ± 7 | 22 ± 5 | 0.38 |
| PR interval (ms) | 178 (146–232) | 312 (204–430) | 182 (158–212) | 0.03 |
| QRS width (ms) | 90 (80–100) | 115 (93–123) | 86 (78–95) | 0.003 |
Values are given as mean ± SD, numbers with percentages or median with IQR. Time of censor for patients with VA is timing of VA (echocardiographic and ECG findings ± 12 months). Time of censor for patients without VA is last clinical evaluation.
ACE, angiotensin converting enzyme; AT2, angiotensin 2 receptor blocker; AV block, atrioventricular block; GLS, global longitudinal strain; i, indexed value; IQR, interquartile range; LAV, left atrial volume; LV EDV, left ventricular end-diastolic volume; LVEF, LV ejection fraction; LV ESV, LV end-systolic volume; MCRA, mineral corticoid receptor antagonist; NYHA, New York Heart Association; NSVT, non-sustained ventricular tachycardia; RVD, right ventricular diameter basal; SD, standard deviation; TAPSE, tricuspid annular plane systolic excursion; VA, ventricular arrhythmia.
AV block degree 1, 2, or 3.